Magnetic Resonance Imaging presentation of Arrhythmogenic Right Ventricular Dysplasia: a case report

Reprint requests to: Dr. Wen-Sheng Tzeng Department of Diagnostic Radiology, Chi-Mei Medical Center, Liou-Ying Campus. No. 201, Taikang Village, Liou-Ying, Tainan 736, Taiwan, R.O.C. Arrhythmogenic right ventricular dysplasia (A RV D) i s a non i schem ic c ard iomyopathy involving primarily the right ventricle. Its characteristics include hypokinetic areas involving the free wall of the right ventricle, fibrofatty replacement of the right ventricular myocardium, and associated arrhythmia originating in the right ventricle. Genetically, it is a cardiomyopathy with autosomal dominant inheritance, variable expressivity, and incomplete penetrance [1]. The location of the gene was mapped to 14q23-24 recently [1]. The incidence and frequency of this disease are still uncertain till now. Clinically, it is difficult to diagnose ARVD directly and accurately. Here, we report a case of 17 years old female with ARVD; the magnetic resonance imaging (MRI) revealed severe global dilatation of the right ventricle, and diffuse thinning of right ventricular myocardium with transmural replacement by fatty tissue and we conclude that MRI may play an important role in diagnosis of the disease. Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) is a rare disease and may lead to sudden death in young adolescents, while old patients are more likely to present with cardiac failure. It is characterized by the patchy replacement of myocardium by fatty or fibrofatty tissue. These changes may lead to structural abnormalities including right ventricular enlargement and wall motion abnormalities that can be detected by echocardioagraphy, angiography, and cine MRI. Here we present a case with the symptom of exertional dyspnea and intermittent tachycardia. The etiology, clinical character ist ics, and image f indings are discussed.